We present for the first time a patient, who from his first months of life developed several autoimmune endocrine and non disorders such as type 1 diabetes, autoimmune thyroiditis, and vitiligo [the constellation of polyglandular autoimmune syndrome IIIA (PG IIIA) manifestations], severe osteoporosis, as well as celiac disease (CD) diagnosed at the age of 9 years-old. Corticosteroids were administered in addition to gluten-free diet, because of partial response, for two decades. During the last 3 years he developed finger clubbing and progressive dyspnea on exertion and was hospitalized repeatedly because of lower respiratory tract infections. The persisting and aggravating chronic dyspnea and diffuse interstitial pattern on chest imaging, led to extensive investigation in order to further characterize his lung disease, disclosing usual interstitial pneumonia (UIP) histologic type lung involvement. On his last admission in our department because of fever and severe deterioration of respiratory status, the patient developed acute lung injury (ALI)/acute respiratory distress syndrome (ARDS), the so called UIP “acute exacerbation”, ending to death.